# Plexiform Neurofibroma of the Vulva in a Patient With Neurofibromatosis Type 1

## TL;DR

A 16-year-old girl with neurofibromatosis type 1 developed a rare plexiform neurofibroma on her vulva, requiring surgery and highlighting the need for early diagnosis and multidisciplinary care.

## Contribution

This case report highlights the rare occurrence of vulvar plexiform neurofibroma in NF-1 and emphasizes the importance of multidisciplinary management.

## Key findings

- Vulvar plexiform neurofibroma is exceptionally rare in NF-1 patients.
- Surgical resection was performed twice due to tumor recurrence.
- A multidisciplinary approach is essential for managing psychosocial and functional challenges.

## Abstract

Plexiform neurofibroma (PNF) is a rare but pathognomonic tumour associated with neurofibromatosis type 1 (NF-1), an autosomal dominant neurocutaneous disorder. Although PNFs can affect various body regions, vulvar involvement is exceptionally uncommon. This report describes a 16-year-old female patient who presented at a dermatology center with a painless, hanging mass on the right labia majora, accompanied by multiple skin café-au-lait spots, freckling, and cutaneous neurofibromas. Clinical evaluation, genetics testing, imaging, and histopathological analysis confirmed a diagnosis of labia majora PNF. Surgical resection was performed twice due to recurrence. Given the psychosocial and functional challenges, including gait limitation, embarrassment, and psychological distress, associated with this rare presentation, a multidisciplinary approach with an individualised management plan and long-term follow-up is essential. This case emphasises the need to raise awareness of vulvar neurofibromas in NF-1 patients to ensure timely diagnosis and intervention.